Written for pulmonary clinicians and scientists alike, Molecular Basis of Lung Disease: Insights from Rare Disorders is a comprehensive and invaluable nesource that sheds new light on the molecular mechanisms influencing the clinical presentation and [books.google.de]

(OI) PowerPoint Presentation: A-aDO2 PAO2-Pao2 Normal range in a new born :5-15 Abnormal :15-40 Definitely abnormal:>40 PowerPoint Presentation: a/A Ratio Ratio of Pao2 to PAO2 It is a better indicator of gas exchange as the ratio is less affected by [authorstream.com]

We present a patient with a novel point mutation resulting in hereditary Surfactant Protein-B (SP-B) deficiency. [content.iospress.com]

Abstract We report on a term newborn with an unusual presentation and course of a rare lung disease due to mutation in SFTPC gene. [nature.com]

SP-B mRNA was present in all lung samples except that of the case patient, in which no signal was detected even after prolonged exposure. [nejm.org]

• Respiratory Insufficiency

  Brasch et al. 7 reported a 13-month-old child with severe respiratory insufficiency associated with de novo missense mutation of SFTPC. [nature.com]

  In addition to causing respiratory insufficiency, RDS increases risk of intraventricular hemorrhage, tension pneumothorax, bronchopulmonary dysplasia, sepsis, and death. [msdmanuals.com]

  In the largest randomized trial completed to date, the OSIRIS (Open Study of Infants at High Risk of or With Respiratory Insufficiency - The Role of Surfactant) Collaborative Study Group enrolled several thousand babies in a protocol to assess when Exosurf [healio.com]

• Short Arm

  SFTPC gene is short, spanning only 3500 base pairs on the short arm of chromosome 8 (8p23.1) near the gene coding for bone morphogenetic protein 1. [nature.com]

[…] suggested by basic research and follows an easy to read format: brief introduction followed by discussion of epidemiology, genetic basis and molecular pathogenesis, animal models, clinical presentation, diagnostic approaches, conventional management and treatment [books.google.de]

Standard Therapies Treatment of infant respiratory distress syndrome consists of mechanical or physical breathing assistance such as positive end expiratory pressure (PEEP). Other treatment is symptomatic and supportive. [rarediseases.org]

which became a more established treatment modality. [healio.com]

Intratracheal surfactant Supplementary oxygen as needed Mechanical ventilation as needed Specific treatment of RDS is intratracheal surfactant therapy. [msdmanuals.com]

Inhalation surfactant therapy in the integrated treatment of severe COVID-19 pneumonia. Tuberculosis and Lung Diseases. 2020; 98 (9): 6–12. 43. Lachmann B. [panor.ru]

Prognosis with treatment is excellent; mortality is 10%. With adequate ventilatory support alone, surfactant production eventually begins, and once production begins, RDS resolves within 4 or 5 days. [msdmanuals.com]

Finally, the prognosis for infants with congenital alveolar proteinosis has been uniformly poor, even with the use of extracorporeal membrane oxygenation. Therapeutic whole-lung lavage has also been considered 4. [nejm.org]

Acute respiratory distress syndrome: how to optimize oxygen transport and to improve prognosis. Ter Arkh. 2014; 86 (11): 115–122. 20. Davidson W. J., Dorscheid D., Spragg R. et al. [panor.ru]

Transient tachypnea Pneumonia Aspiration syndromes Pneumothorax Pulmonary hypoplasia Pleural effusions Airway problems Hyaline membrane disease Pneumonia Pulmonary hemorrhage Air leak syndromes Pulmonary hypoplasia Bronchopulmonary dysplasia Laryngomalacia Etiology [authorstream.com]

The etiology of BPD is problematic and may be due to oxygen, overventilation or underventilation. [en.wikipedia.org]

It is occasionally difficult to distinguish respiratory from nonrespiratory etiologies on the basis of clinical signs alone. [clinicalgate.com]

Thus, despite similar chest x-rays and histopathological findings, the BAL profile for SP-B, SP-C and their proforms appears useful in distinguishing PAP from SP-B deficiency of any etiology. [respiratory-research.biomedcentral.com]

Each chapter focuses on the pathogenic mechanisms and therapeutic targets suggested by basic research and follows an easy to read format: brief introduction followed by discussion of epidemiology, genetic basis and molecular pathogenesis, animal models [books.google.de]

Relevant External Links for SFTPB Genetic Association Database (GAD) SFTPB Human Genome Epidemiology (HuGE) Navigator SFTPB Atlas of Genetics and Cytogenetics in Oncology and Haematology: SFTPB No data available for Genatlas for SFTPB Gene Polymorphic [genecards.org]

HMD - Pathophysiology: HMD - Pathophysiology Terminal airspaces are not uniform throughout the lung There is a range of critical opening and critical closing pressures in various alveoli Lung Function in HMD: Lung Function in HMD Lung Compliance is also [authorstream.com]

This chapter describes the pathophysiology and molecular biology of hereditary SP-B deficiency. [ommbid.mhmedical.com]

Which of the following pathophysiologic conditions are components of meconium aspiration? I. Surfactant inactivation II. [studystack.com]

Pathophysiology of respiratory distress syndrome. The above scenario is repeated nightly throughout the United States with private pediatricians caring for such infants on a regular basis. [healio.com]

Pathophysiology [ edit ] The lungs of infants with respiratory distress syndrome are developmentally deficient in a material called surfactant, which helps prevent collapse of the terminal air-spaces (the future site of alveolar development) throughout [en.wikipedia.org]

Extensive testing of each preparation has demonstrated that both products are efficacious in preventing and treating RDS and preventing the complications of pulmonary airleak. [healio.com]

The only way to prevent the need for surfactant replacement therapy is to prevent a premature birth. [medical-dictionary.thefreedictionary.com]

Timely and appropriate therapy is essential to prevent ongoing injury and improve outcome. [clinicalgate.com]

Figure 5 The airspaces are lined with a lipoprotein complex called pulmonary surfactant, named for its ability to maintain normal respiratory mechanics by reducing surface tension at the air/liquid interface, preventing the lungs from collapsing at the [webs.ucm.es]

Prevention [ edit ] Giving the mother glucocorticoids speeds the production of surfactant. For very premature deliveries, a glucocorticoid is given without testing the fetal lung maturity. [en.wikipedia.org]